Malignant peripheral nerve sheath tumor (MPNST) arising from angiosarcoma: a rare case report.

Purpose: An extremely rare case of divergent differentiation of a cutaneous angiosarcoma to a malignant peripheral nerve sheath tumor (MPNST) is presented.

Methods: A 62-year-old male patient presented with an angiosarcoma of the left buccal region, which was excised with wide surgical margins. Four months after the operation, the tumor recurred locally as a MPNST. The recurrent tumor was treated with surgical excision followed by postoperative radiotherapy. Four years later, the patient presented with solitary lung metastasis. The patient underwent upper right lobectomy.

Results: The patient is alive and disease-free 2 years after the lobectomy and 6 years after initial presentation.

Conclusions: Divergent differentiation of an angiosarcoma to a neurosarcoma supports the theory that cells of the neural crest capable of differentiating both to neuroectoderm and to mesenchyme give origin to these tumors.